Definition:

Subacute sclerosing panencephalitis (SSPE) is a progressive, fatal neurologic disorder of childhood and early adolescence. It is caused by a persistent infection of the brain by an aberrant measles virus. Signs attributable to subacute sclerosing panencephalitis usually develop 4-8 years after the onset of measles infection. It is not precisely known how the measles virus remains dormant for so many years and why it becomes active again. More recent data indicate that it can follow acute measles infection in 1:10,000 cases. The incidence of SSPE is inversely related to measles vaccination coverage. In the developed world, the prevalence of SSPE has steadily declined since the introduction of the measles virus vaccine. The characteristic clinical manifestations of SSPE include behavioral abnormalities, cognitive decline, myoclonic jerks, seizures and abnormalities in vision. Clinical manifestations of SSPE usually start with subtle intellectual deterioration of the affected child causing deterioration in school performance. One of the most characteristic manifestations of SSPE is periodic myoclonic jerks that often leads to difficulty in walking and repeated falls. In the terminal stages of the disease, decerebrate and decorticate rigidity appear. Ultimately the patient becomes vegetative. Management of the disease includes seizure control and avoidance of secondary complications associated with the progressive disability. Treatment with interferon, ribavirin, and isoprinosine have reported beneficial results. However, there is no definitively effective treatment for SSPE.